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Article

Metabolic Impact of Rapamycin (Sirolimus) and B-Estradiol Using Mouse Embryonic Fibroblasts as a Model for Lymphangioleiomyomatosis.

Lung - 1 Aug 2017

Marsh Katherine M, Schipper David, Ferng Alice S, Johnson Kitsie, Fisher Julia, Knapp Shannon, Dicken Destiny, Khalpey Zain

Abstract excerpt

INTRODUCTION: Lymphangioleiomyomatosis (LAM) is a rare, progressive cystic lung disease that predominantly affects women of childbearing age. Exogenous rapamycin (sirolimus) has been shown to improve clinical outcomes and was recently approved to treat LAM, whereas estrogen (E2) is implicated in disease progression. No consistent metabolic model currently exists for LAM, therefore wild-type mouse embryonic...

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PMID: 28577037
DOI: 10.1007/s00408-017-0016-3

Genes

TSC2

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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